& Cluster Headache



• Migraine headache (HA): A chronic, recurrent episodic, stereotypical HA syndrome not caused by intracranial or systemic disease (but neural-based)

– ± associated migraine features; ± aura

– International Headache Society (IHS): Diagnostic criteria and migraine subtypes: Migraine without aura →

A. At least 5 attacks fulfilling B–D

B. HA lasting for 4–72 hours

C. HA with at least 2 of the following: Unilateral location, pulsing quality, moderate to severe intensity, aggravation by or avoidance of routine activity

D. HA accompanied by at least 1 of following: Nausea and/or vomiting, photophobia, or phonophobia

E. Not attributed to another disorder

• Cluster HA: Episodic, short-lived attacks, clustered temporally, followed by pain-free interval.

– IHS criteria:

– A. 5 attacks fulfilling B–D

– B. Severe, unilateral, orbital, supraorbital, or temporal pain lasting 15–80 minutes

– C. At least 1 ipsilateral feature: Conjunctival injection/lacrimation, nasal congestion/rhinorrhea, eyelid edema, forehead/facial sweating, ptosis, miosis, restlessness or agitation

– D. Frequency: every other day to 8 attacks/day

– E. Not attributed to another disorder

– Characteristic circadian & circannual features

• Initial determinations: 1) Primary (no underlying intracranial or systemic disease) versus secondary HA 2) New onset versus chronic

– Requires emergent evaluation (e.g., neuroimaging ± lumbar puncture (LP)):

– Acute onset, maximal at onset or focal signs; Progressive symptoms and signs; systemic symptoms (e.g., fever)

– Other worrisome features: Onset fourth to fifth decade, accelerating pattern, history of malignancy or HIV, significant change in HA pattern, exacerbation with position change, exertion, sexual activity, or Valsalva



Migraine: ♀:♂ 3:1; cumulative lifetime: ♀ 43%, ♂ 18%. cluster: ♂:♀ 8:1


Migraine 12–15%; cluster ∼69/100,000


Family history: ↑ Risk by 50% versus controls


Familial hemiplegic migraine 1, 19p13


Identification and avoidance of migraine triggers


Central activation of pain sensitive cranial structures; serotonergic neurotransmission dysfunction; central pain sensitization


Hereditary; pattern and mode of inheritance unclear; multiple genes contribute


Depression, panic disorder, epilepsy, asthma, non-HA pain



• See IHS criteria

• Assess: Inciting event, new onset/de novo versus chronic/recurrent, quality, duration, location, and time course

– Characteristic frequency and symptom pattern?

– Exacerbating or alleviating factors? Triggers?

– Position, posture, or Valsalva related?

– Age >50 years: Temporal arteritis ROS?

– Time-intensity relationship:

– Subarachnoid hemorrhage (SAH): Maximal intensity at onset, that is, “thunder-clap”

– Cluster: Peaks over 3–5 minutes, maximal for 1–2 hours, tapers off.

– Migraine: Escalates over hour(s), lasts hours to days.

– Migraine features? Aura, nausea, vomiting, photophobia, and phonophobia

– Cluster: Excruciating, acute, unilateral periorbital pain; crescendo within 5 minutes; ipsilateral autonomic features

Pathognomonic: Provoked by ETOH in ∼70%

• Assess additional symptoms: Transient visual obscurations, vertigo, meningismus, amenorrhea, galactorrhea, fever, purulent nasal discharge, myalgias, scalp tenderness, jaw claudication, and cognitive dysfunction.

– History of focal neurological symptoms?

• Past medical, psychiatric, and medication history: Neoplasm, aneurysm, or HIV history?


Complete neurological exam including funduscopy: Normal except for transient aura signs.



Initial lab tests

Exclusion of secondary causes: Metabolic panel, CBC/Platelets, ESR and CRP for age >50 years

Follow-up & special considerations

ANA, Anti-DS DNA Ab, Arterial blood gas, sleep study to r/o sleep apnea, anti TSH-receptor Ab, → tailor to individual patient


Initial approach

• New onset HA of non-urgent nature: MRI

• Acute, severe HA, or focal signs: Urgent non-contrast CT

Follow-up & special considerations

• MRV for venous sinus thrombosis and work-up of idiopathic intracranial hypertension (IIH)

• CTA or MRI/A with axial T1 fat suppression: Optimal for ruling out dissection

Diagnostic Procedures/Other


• LP must be obtained for suspected SAH if CT negative (prior to MRI)

• Visual aura: Include visual field to exclude homonymous defects indicating parenchymal disease

• Clinical impact scales: MIDAS, HIT, MIBS, MSQ, MPQ-5

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Nov 9, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on & Cluster Headache

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